Radiofrequency ablation via catheter and transpapillary access in patients with cholangiocarcinoma (ACTICCA-2 trial) - a multicenter, randomized, controlled, open-label investigator-initiated trial.

BACKGROUND: Despite the recent advances in cancer treatment, the therapeutic options for patients with biliary tract cancer are still very limited and the prognosis very poor. More than 50% of newly diagnosed patients with biliary tract cancer are not amenable to curative surgical treatment and thus treated with palliative systemic treatment. Malignant bile duct obstructions in patients with perihilar and/or ductal cholangiocarcinoma (CCA) represents one of the most important challenges in the management of these patients, owning to the risk represented by developing life-threatening cholangitis which, in turn, limits the use of systemic treatment. For this reason, endoscopic stenting and/or bile duct decompression is the mainstay of treatment of these patients. Data on efficacy and safety of adding radiofrequency ablation (RFA) to biliary stenting is not conclusive. The aim of this multicenter, randomized trial is to evaluate the effect of intraductal RFA prior to bile duct stenting in patients with unresectable perihilar or ductal CCA undergoing palliative systemic therapy. METHODS/DESIGN: ACTICCA-2 is a multicenter, randomized, controlled, open-label, investigator-initiated trial. 120 patients with perihilar or ductal CCA with indication for biliary stenting and systemic therapy will be randomized 1:1 to receive either RFA plus bile duct stenting (interventional arm) or bile duct stenting alone (control arm). Patients will be stratified by trial site and tumor location (perihilar vs. ductal). Both arms receive palliative systemic treatment according to the local standard of care determined by a multidisciplinary tumorboard. The primary endpoint is time to first biliary event, which is determined by an increase of bilirubin to > 5 mg/dl and/or the occurrence of cholangitis leading to premature stent replacement and/or disruption of chemotherapy. Secondary endpoints include overall survival, safety according to NCI CTCAE v5, quality of life assessed by questionnaires (EORTC QLQ-C30 and QLQ-BIL21), clinical event rate at 6 months after RFA and total days of over-night stays in hospital. Follow-up for the primary endpoint will be 6 months, while survival assessment will be continued until end of study (maximum follow-up 30 month). All patients who are randomized and who underwent endoscopic stenting will be used for the primary endpoint analysis which will be conducted using a cause-specific Cox proportional hazards model with a frailty for trial site and fixed effects for the treatment group, tumor location, and stent material. DISCUSSION: ACTICCA-2 is a multicenter, randomized, controlled trial to assess efficacy and safety of adding biliary RFA to bile duct stenting in patients with CCA receiving palliative systemic treatment. TRIAL REGISTRATION: The study is registered with ClinicalTrials.gov (NCT06175845) and approved by the local ethics committee in Hamburg, Germany (2024-101232-BO-ff). This manuscript reflects protocol version 1 as of January 9th, 2024.

Initial clinical outcomes of robotic resection for perihilar cholangiocarcinoma: Is it safe and effective?

BACKGROUND AND OBJECTIVES: We aimed to describe our outcomes of robotic resection for perihilar cholangiocarcinoma, the largest single institutional series in the Western hemisphere to date. METHODS: Between 2016 and 2022, we prospectively followed all patients who underwent robotic resection for perihilar cholangiocarcinoma. RESULTS: In total, 23 patients underwent robotic resection for perihilar cholangiocarcinoma, 18 receiving concomitant hepatectomy. The median age was 73 years. Operative time was 470 min with an estimated blood loss of 150 mL. No intraoperative conversions to open or other intraoperative complications occurred. Median length of stay was 5 days. Four postoperative complications occurred. Three readmissions occurred within 30 days with one 90-day mortality. R0 resection was achieved in 87% of patients and R1 in 13% of patients. At a median follow-up of 27 months, 15 patients were alive without evidence of disease, two patients with local recurrence at 1 year, and six were deceased. CONCLUSIONS: Utilization of the robotic platform for perihilar cholangiocarcinoma is safe and feasible with excellent perioperative outcomes. Further studies are needed to determine the long-term oncological outcomes.

Preoperative predictors for non-resectability in perihilar cholangiocarcinoma.

INTRODUCTION: Explorative laparotomy without subsequent curative-intent liver resection remains a major clinical problem in the treatment of perihilar cholangiocarcinoma (pCCA). Thus, we aimed to identify preoperative risk factors for non-resectability of pCCA patients. MATERIAL AND METHODS: Patients undergoing surgical exploration between 2010 and 2022 were eligible for the analysis. Separate binary logistic regressions analyses were used to determine risk factors for non-resectability after explorative laparotomy due to technical (tumor extent, vessel infiltration) and oncological (peritoneal carcinomatosis, distant nodal or liver metastases)/liver function reasons. RESULTS: This monocentric cohort comprised 318 patients with 209 (65.7%) being surgically resected and 109 (34.3%) being surgically explored [explorative laparotomy: 87 (27.4%), laparoscopic exploration: 22 (6.9%)]. The median age in the cohort was 69 years (range 60-75) and a majority had significant comorbidities with ASA-Score ≥ 3 (202/318, 63.5%). Statistically significant (p < 0.05) risk factors for non-resectability were age above 70 years (HR = 3.76, p = 0.003), portal vein embolization (PVE, HR = 5.73, p = 0.007), and arterial infiltration > 180° (HR = 8.05 p < 0.001) for technical non-resectability and PVE (HR = 4.67, p = 0.018), arterial infiltration > 180° (HR = 3.24, p = 0.015), and elevated CA 19-9 (HR = 3.2, p = 0.009) for oncological/liver-functional non-resectability. CONCLUSION: Advanced age, PVE, arterial infiltration, and elevated CA19-9 are major risk factors for non-resectability in pCCA. Preoperative assessment of those factors is crucial for better therapeutical pathways. Diagnostic laparoscopy, especially in high-risk situations, should be used to reduce the amount of explorative laparotomies without subsequent liver resection.

Survival analysis of laparoscopic surgery and open surgery for hilar cholangiocarcinoma: a retrospective cohort study.

BACKGROUND/PURPOSE: This study compared the clinical efficacy and safety of laparoscopic versus open resection for hilar cholangiocarcinoma (HCCA) and analyzed potential prognostic factors. METHODS: The study included patients who underwent HCCA resection at our center from March 2012 to February 2022. Perioperative complications and postoperative prognosis were compared between the laparoscopic surgery (LS) and open surgery (OS) groups. RESULTS: After screening 313 HCCA patients, 68 patients were eligible for the study in the LS group (n = 40) and OS group (n = 28). Kaplan-Meier survival curve analysis revealed that overall survival > 2 years and 3-year disease-free survival (DFS) were more common in the LS than OS group, but the rate of 2-year DFS was lower in the LS group than OS group. Cox multivariate regression analysis revealed age (< 65 years), radical resection, and postoperative adjuvant therapy were associated with reduced risk of death (hazard ratio [HR] = 0.380, 95% confidence interval [CI] = 0.150-0.940, P = 0.036; HR = 0.080, 95% CI = 0.010-0.710, P = 0.024 and HR = 0.380, 95% CI = 0.150-0.960, P = 0.040), whereas preoperative biliary drainage was an independent factor associated with increased risk of death (HR = 2.810, 95% CI = 1.130-6.950, P = 0.026). Perineuronal invasion was identified as an independent risk factor affecting DFS (HR = 5.180, 95% CI = 1.170-22.960, P = 0.030). CONCLUSIONS: Compared with OS, laparoscopic HCCA resection does not significantly differ in terms of clinical efficacy. Age (<65 years), radical resection, and postoperative adjuvant therapy reduce the risk of death, and preoperative biliary drainage increases the risk of death.

[Surgical treatment and prognostic factors in perihilar tumors]. / Khirurgicheskoe lechenie i faktory prognoza pri perikhilyarnoi opukholi.

OBJECTIVE: To study the results of surgical treatment in patients with perihilar tumors. MATERIAL AND METHODS: We analyzed 98 patients with perihilar tumors who underwent surgery. RESULTS: We prefer percutaneous transhepatic biliary drainage (n=58) for jaundice. Retrograde interventions were performed in 18 cases (20.5%), complications grade III-IV were more common (p=0.037) in the last group. Postoperative mortality was 12%. Complications developed in 81 patients (82.7%), grade ≥3 - in 39 (39.8%) cases. Portal vein resection (n=26) increased the incidence of complications grade ≥III (p=0.035) and portal vein thrombosis (p=0.0001). Chemotherapy after surgery was performed in 47 patients (48.0%), photodynamic therapy - in 7 (7.1%) patients. A 5-year overall survival was 28.1%, the median survival - 29 months. R2 resection and/or M1 stage (n=12) significantly worsened the prognosis and overall survival (16.5 vs. 31 months, p=0.0055). Lymph node (LN) lesion, microscopic status (R0 vs. R1) of resection margin, technique of decompression and isolated resection of extrahepatic bile ducts did not affect the prognosis, and we combined appropriate patients (n=72) for analysis. SI resection and excision of ≥6 lymph nodes were independent positive factors for disease-free survival (p=0.042 and p=0.007, respectively). Blood transfusion and high preoperative neutrophil-lymphocyte index (NLI ≥2.15) worsened overall (p=0.009 and p=0.002, respectively) and disease-free survival (p=0.002 and 0.007, respectively). The absence of adjuvant therapy worsened disease-free survival alone (p=0.024). CONCLUSION: SI liver resection, adequate lymph node dissection and adjuvant therapy should be used for perihilar tumors. Isolated resection of extrahepatic bile ducts is permissible in some cases. Blood transfusion and NLI ≥2.15 are independent negative prognostic factors.

Percutaneous endobiliary radiofrequency ablation with stent placement in type IV hilar cholangiocarcinoma: A prospective comparison with stent placement alone.

OBJECTIVES: This study aimed to compare the safety and effectiveness of percutaneous endobiliary radiofrequency ablation with stent placement (RFA group) versus stent placement alone (stent group) in patients with type IV hilar cholangiocarcinoma. METHODS: This prospective nonrandomized study was conducted between October 2021 and April 2023. The study included 56 participants (33 men and 23 women, median age 73 years) who underwent percutaneous endobiliary RFA with stent placement (n = 25) or stent placement alone (n = 31) for type IV hilar cholangiocarcinoma. The primary end point was stent patency, while the secondary end points were procedure-related adverse events (AE) and overall survival. RESULTS: The percutaneous endobiliary RFA and/or stent placement were successfully completed in all patients in both groups. The median stent patency rate was higher in the RFA group than the stent group (188 days vs. 155 days, p = 0.048). There were no differences in AEs (grade 1 [5 in RFA group vs. 5 in stent group, p = 0.74] and grade 2 AEs [2 vs. 4, p = 0.68]) and patients' survival (median 222 days vs. 214 days, p = 0.49) between the two groups. CONCLUSIONS: In patients with type IV hilar cholangiocarcinoma, percutaneous endobiliary RFA with stent placement may improve stent patency without increasing the risk of AEs compared to stent placement alone.

Ex vivo liver resection and auto-transplantation and special systemic therapy in perihilar cholangiocarcinoma treatment.

This editorial contains comments on the article "Systematic sequential therapy for ex vivo liver resection and autotransplantation: A case report and review of literature" in the recent issue of World Journal of Gastrointestinal Surgery. It points out the actuality and importance of the article and focuses primarily on the role and place of ex vivo liver resection and autotransplantation (ELRAT) and systemic therapy, underlying molecular mechanisms for targeted therapy in perihilar cholangiocarcinoma (pCCA) management. pCCA is a tough malignancy with a high proportion of advanced disease at the time of diagnosis. The only curative option is radical surgery. Surgical excision and reconstruction become extremely complicated and not always could be performed even in localized disease. On the other hand, ELRAT takes its place among surgical options for carefully selected pCCA patients. In advanced disease, systemic therapy becomes a viable option to prolong survival. This editorial describes current possibilities in chemotherapy and reveals underlying mechanisms and projections in targeted therapy with kinase inhibitors and immunotherapy in both palliative and adjuvant settings. Fibroblast grow factor and fibroblast grow factor receptor, human epidermal growth factor receptor 2, isocitrate dehydrogenase, and protein kinase cAMP activated catalytic subunit alpha (PRKACA) and beta (PRKACB) pathways have been actively investigated in CCA in last years. Several agents were introduced and approved by the Food and Drug Administration. They all demonstrated meaningful activity in CCA patients with no global change in outcomes. That is why every successfully treated patient counts, especially those with advanced disease. In conclusion, pCCA is still hard to treat due to late diagnosis and extremely complicated surgical options. ELRAT also brings some hope, but it could be performed in very carefully selected patients. Advanced disease requires systemic anticancer treatment, which is supposed to be individualized according to the genetic and molecular features of cancer cells. Targeted therapy in combination with chemo-immunotherapy could be effective in susceptible patients.

[Non-Hodgkin large B-cell lymphoma of the bile duct mimicking a Klatskin tumor: case report]. / Linfoma no Hodgkin de células B grandes de la vía biliar simulando un tumor de Klatskin: relato de caso.

The obstruction of the bile duct secondary to non-Hodgkin lymphoma is extremely rare. That's why we present the case of a 63-year-old female patient who sought medical attention due to jaundice, dark urine, acholia, and weakness. Laboratory results showed a cholestatic pattern, and an ultrasound revealed dilation of the intra and extrahepatic bile ducts, for which a cholangio resonance was ordered. It showed an expansive formation with ill-defined borders compromising the common hepatic duct associated with its stenosis. The initial suspicion was a Klatskin tumor, for which a biopsy was performed, which reported infiltration of a double expressor large B-cell lymphoma as a primary neoplasm of the bile duct. The patient underwent chemotherapy treatment with R-CHOP regimen (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) and went into remission. Due to continuous episodes of cholangitis, a Roux-en-Y hepatic jejunal anastomosis with biliary tract reconstruction was performed. Currently, she remains in remission, seven years after the diagnosis. This case highlights the rarity of large B-cell non-Hodgkin lymphoma in the bile duct and emphasizes the importance of biopsy for effective treatment, combining chemotherapy for the underlying disease and surgery for obstructive complications.

La obstrucción de la vía biliar secundaria a un linfoma no hodgkin es extremadamente raro. Es por esto que presentamos el caso de una paciente femenina de 63 años que consulta por ictericia, coluria, acolia y astenia. Un laboratorio presentando un patrón colestásico y una ecografía con la vía biliar intra y extrahepática dilatadas llevaron a realizar una colangioresonancia de abdomen que evidenció una formación expansiva de limites mal definidos que comprometía el conducto hepático común asociado a estenosis del mismo. La sospecha inicial fue un tumor de klatskin y se llevó a cabo la toma de biopsia, cuyo resultado anatomopatológico informó infiltración de linfoma de células B de células grandes doble expresor como tumor primario de la vía biliar. Realizó tratamiento quimioterápico con esquema R CHOP (rituximab, ciclofosfamida, doxorrubicina, vincristina, prednisona) y entró en remisión. Por continuos episodios de colangitis se optó por realizar una hepático yeyuno anastomosis en Y de Roux con reconstrucción de la vía biliar. Actualmente continúa en remisión a 7 años del diagnóstico. El caso resalta la rareza del linfoma no hodgkin de células B grandes en la vía biliar, y destaca la importancia de la biopsia para un tratamiento eficaz que combina la quimioterapia para la enfermedad de base y la cirugía para las complicaciones obstructivas.

Neurofibroma de la vía biliar: una causa rara de ictericia colestásica que imita el tumor de Klatskin

El neurofibroma de la vía biliar es una enfermedad extremadamente rara y generalmente asintomática, sin embargo, puede ocasionar ictericia obstructiva y simular un tumor de Klatskin conduciendo a un tratamiento quirúrgico radical y mayor morbilidad del paciente. Presentamos el caso de una mujer de 62 años colecistectomizada hace 10 años, con ictericia y dolor en hipocondrio derecho, así como exámenes auxiliares compatibles con colestasis. Se observó dilatación de la vía biliar y presencia de un tumor en el conducto hepático izquierdo mediante colangioresonancia magnética. El diagnóstico clínico preoperatorio fue Colangiocarcinoma hiliar, pero el estudio histopatológico reveló una neoplasia compuesta por células fusocelulares sin atipia ni actividad mitótica, cuya estirpe neurogénica se sustentó por su positividad a proteína S100 en inmunohistoquímica. Reportamos el caso dada su poca frecuencia en la literatura y su relevancia, al ser una entidad benigna, como diagnóstico diferencial de cáncer.

The neurofibroma of the bile duct is an extremely rare and generally asymptomatic disease, however, it maybe cause obstructive jaundice and mimic a Klatskin tumor, leading to radical surgical treatment and increased patient morbidity. We present the case of a 62-year-old woman who underwent cholecystectomy 10 years ago, with jaundice and pain in the right upper quadrant, as well as auxiliary tests compatible with cholestasis. Dilation of the bile duct and the presence of a tumor in the left hepatic duct were observed by magnetic cholangioresonance. The preoperative clinical diagnosis was hilar cholangiocarcinoma, but the histopathological study revealed a neoplasm composed of spindle cells without atypia or mitotic activity, whose neurogenic lineage supported by its positivity to protein S100 in immunohistochemistry. We report the case given its infrequency in the literature and its relevance, as it is a benign entity, as a differential diagnosis of cancer.

Protocol for liver transplantation in hilar cholangiocarcinoma / Protocolo de transplante hepático para colangiocarcinoma hilar

ABSTRACT Background: Hilar cholangiocarcinoma represents more than half of all cholangiocarcinoma cases, having poor prognosis and presenting a median overall survival after diagnosis of 12-24 months. In patients who have unresectable tumors with a better prognosis, the proposal to perform liver transplantation emerged for expanding the possibility of free margins by performing total hepatectomy. Aim: To provide a Brazilian protocol for liver transplantation in patients with hilar cholangiocarcinoma. Method: The protocol was carried out by two Brazilian institutions which perform a large volume of resections and liver transplantations, based on the study carried out at the Mayo Clinic. The elaboration of the protocol was conducted in four stages. Result: A protocol proposal for this disease is presented, which needs to be validated for clinical use. Conclusion: The development of a liver transplantation protocol for cholangiocarcinoma aims not only to standardize the treatment, but also enable a better assessment of the surgical results in the future.

RESUMO Racional: O colangiocarcinoma hilar representa mais da metade de todos os casos de colangiocarcinoma; tem prognóstico reservado e sobrevida global mediana de 12- 24 meses após o diagnóstico. A proposta de realizar transplante hepático surgiu para ampliar a possibilidade de margens livres através de hepatectomia total nos portadores de tumoresirressecáveis com melhor prognóstico. Objetivo: Apresentar protocolo brasileiro para realização de transplante hepático em pacientes com colangiocarcinoma hilar. Método: O protocolo foi realizado por duas instituições com grande volume de ressecções e transplantes hepáticos no Brasil, baseado no trabalho realizado pela MayoClinic. A elaboração foi dividida em quatro etapas. Resultado: É apresentada proposta de protocolo para esta doença a ser validada na aplicação clínica. Conclusão: Foi possível elaborar protocolo de transplante hepático para colangiocarcinoma a fim de uniformizar o tratamento e melhor avaliar os resultados cirúrgicos.

Tumor de Klatskin en una paciente con diagnóstico de síndrome de Sjögren / Klatskin tumor in a patient diagnosed with Sjögren's syndrome

El proceso inflamatorio crónico de las enfermedades reumáticas constituye un factor que causa afectación en otros órganos y sistemas de órganos. La afectación puede llegar a producir metaplasia de la mucosa de distintos tejidos y condicionar la aparición de enfermedades neoplásicas. Los pacientes con síndrome de Sjögren tienen un 40 por ciento de aumento de riesgo de padecer una enfermedad tumoral con respecto a los pacientes sanos o con otras enfermedades crónicas no inflamatorias. El objetivo del presente estudio es dar a conocer las características clínico-imagenológicas que posibilitaron el diagnóstico de un tumor de Klatskin en una paciente femenina de 57 años de edad con antecedentes de 5 años de evolución del síndrome de Sjögren. Se realizó una colangiopancreatografía retrógrada endoscópica para eliminar el tumor. A los tres días de la intervención quirúrgicamente la paciente muestra una evolución favorable por lo que se decide dar alta hospitalaria con seguimiento por consulta externa de especialidades de cirugía general y reumatología indistintamente. Durante todo el procedimiento se mantuvo el tratamiento para la enfermedad de base de la paciente. Actualmente la paciente se encuentra incorporada a sus actividades cotidianas y con una evolución favorable de su estado de salud(AU)

The chronic inflammatory process of rheumatic diseases constitutes a factor that causes affectation in other organs and organ systems. The affectation can produce metaplasia of the mucosa of different tissues and condition the appearance of neoplastic diseases. Patients with Sjögren's syndrome have a 40 percent increased risk of developing a tumor disease compared to healthy patients or other non-inflammatory chronic diseases. The objective of this study is to present the clinical-imaging characteristics that made the diagnosis of a Klatskin tumor possible in a 57-year-old female patient with a 5-year history of Sjögren's syndrome. Endoscopic retrograde cholangiopancreatography was performed to remove the tumor. Three days after the surgical intervention, the patient showed a favorable evolution, so it was decided to discharge from the hospital with follow-up by outpatient consultation of specialties of general surgery and rheumatology indistinctly. Treatment for the patient's underlying disease was maintained throughout the procedure. Currently the patient is incorporated into her daily activities and with a favorable evolution of her state of health(AU)

Tumor de Klatskin (colangiocarcinoma hiliar) su morbimortalidad en un servicio de cirugía / Klatskin's tumor (hilar cholangiocarcinoma) his morbi-mortality in a surgery service

RESUMEN Introducción: El tumor de Klatskin es el colangiocarcinoma, más frecuente de la vía biliar siendo responsable de una alta morbimortalidad en los servicios de cirugía. Objetivo: Determinar la morbilidad y la mortalidad por tumor de Klatskin en el Servicio de Cirugía del Hospital Universitario "Manuel Ascunce Domenech". Métodos: Se realizó un estudio descriptivo, prospectivo y observacional de pacientes que ingresaron en el Servicio de Cirugía General con diagnóstico de tumor de Klatskin, entre septiembre de 2018 y enero del 2020. El universo estuvo conformado por 7 pacientes que cumplieron con los criterios de inclusión. Se utilizaron métodos estadísticos descriptivos y cálculos con valores porcentuales. Resultados: La mayor incidencia de los pacientes fue de sexo masculino y de raza blanca, con un 71,4 por ciento y 85,7 por ciento respectivamente. Predominó adenocarcinoma como variedad histológica con un 85,7 por ciento. Tipo II de la clasificación topográfica, el procedimiento de Hess como operación realizada y la bilirragia como complicación prevaleció con un 42,8 por ciento respectivamente. El 85,7 por ciento de los pacientes egresaron vivos y con una cirugía con finalidad curativa. Conclusiones: La mayoría de los pacientes eran masculinos y de color blanco. Más de la mitad de los pacientes fueron clasificados como tipo I y II según clasificación de Bismuth-Corlette. El proceder de Hess, el adenocarcinoma como forma histológica y el estado del egreso vivo predominó en el total de pacientes(AU)

ABSTRACT Introduction: Klatskin's tumor is cholangiocarcinoma, most frequent to occur in the bile duct, being responsible for high morbidity and mortality in surgery departments. Objective: To determine the morbidity and mortality of Klatskin's tumor at the surgery service of Manuel Ascunce Domenech University Hospital. Methods: We carried out a descriptive, prospective and observational study of patients admitted to the general surgery service with a diagnosis of Klatskin's tumor, between September 2018 and January 2020. The study population consisted of seven patients who met the inclusion criteria. Descriptive statistical methods and calculations with percentage values were used. Results: The highest incidence was represented male and white patients, accounting for 71.4 percent and 85.7 percent, respectively. Adenocarcinoma predominated as a histological variety, accounting for 85.7 percent. There was prevalence of type II of topographic classification, the Hess procedure as the performed operation, and bilirrhagia as a complication, accounting for 42.8 percent, respectively. 85.7 percent of the patients were discharged and received surgery for curative purposes. Conclusions: Most of the patients were male and white. More than half of the patients were classified as types I and II, according to the Bismuth-Corlette classification. The Hess procedure, adenocarcinoma as a histological form, and discharge predominated in all patients(AU)

Elementos etiopatogénicos y diagnósticos del tumor de Klatskin o colangiocarcinoma hiliar / Etiopathogenetic and Diagnostic Elements of Klatskin's Tumor or Hilar Cholangiocarcinoma

RESUMEN Introducción: El colangiocarcinoma hiliar es un tumor poco frecuente, de mal pronóstico y elevada mortalidad; con un curso silente hasta la fase avanzada de la enfermedad. Objetivo: Describir la etiopatogenia y el diagnóstico por imágenes del colangiocarcinoma hiliar. Métodos: De las bases datos PubMed, SciELO y Latindex, se seleccionaron artículos publicados desde 2005 hasta mayo de 2020, relacionados con el colangiocarcinoma hiliar/ perihiliar: etiopatogenia, diagnóstico clínico, estudios de laboratorio y estudios imaginológicos. Desarrollo: Ictericia obstructiva (90 por ciento), pérdida de peso (60 - 75 por ciento) y dolor abdominal (40 por ciento) constituyen los síntomas de presentación más frecuentes. Los factores predisponentes, genéticos y ambientales, desencadenan respuesta inflamatoria crónica que lesionan el DNA de las células ductales provocando diferenciación celular anómala con el desarrollo de colangiocarcinoma. Las infecciones parasitarias y enfermedad litiásica de las vías biliares, en países orientales y la colangitis esclerosante primaria, en occidente, constituyen los principales factores predisponentes. El trípode para el diagnóstico lo conforman: la ecografía, tomografía axial computarizada y la resonancia magnética nuclear. Conclusiones: El colangiocarcinoma hiliar es una causa de colestasis poco frecuente en la población general, con un predominio sexta década de la vida y en el sexo masculino, siendo la ictericia el motivo de consulta de estos pacientes, donde la ecografía y la tomografía axial computarizada juegan el papel más importante en su diagnóstico y donde la clasificación de Bismuth-Corlette y TNM son esenciales para la correcta planificación del tratamiento(AU)

ABSTRACT Introduction: Hilar cholangiocarcinoma is a rare tumor, with poor prognosis and high mortality, with a silent course until the advanced stage of the disease. Objective: To describe the etiopathogenesis and imaging diagnosis of hilar cholangiocarcinoma. Methods: From the PubMed, SciELO and Latindex databases, articles published from 2005 to May 2020 were selected, insofar they were related to hilar/perihilar cholangiocarcinoma: etiopathogenesis, clinical diagnosis, laboratory studies and imaging studies. Development: Obstructive jaundice (90 percent), weight loss (60-75 percent) and abdominal pain (40 percent) are the most frequent presenting symptoms. Predisposing factors, either genetic and environmental, trigger chronic inflammatory responses that damage the DNA of ductal cells, causing abnormal cell differentiation with the development of cholangiocarcinoma. Parasitic infections and bile duct stone disease in Eastern countries and primary sclerosing cholangitis in the West are the main predisposing factors. The tripod for diagnosis is made up of ultrasound, computerized axial tomography and nuclear magnetic resonance. Conclusions: Hilar cholangiocarcinoma is a rare cause of cholestasis among the general population, with a predominance during the sixth decade of life and among males, jaundice being the reason for consultation of these patients, in which ultrasound and computerized axial tomography play the most important elements for its diagnosis, while the TNM and Bismuth-Corlette classification are essential for the correct planning of treatment(AU)

Relationship between serum MMP-9 level and prognosis after radical resection for Hilar cholangiocarcinoma patients

Abstract Purpose: To analyze the preoperative serum matrix metalloproteinase-9 (MMP-9) levels and prognosis of patients with hilar cholangiocarcinoma (HC) undergoing radical resection. Methods: Preoperative serum MMP-9 levels in patients with HC undergoing radical resection were detected by enzyme-linked immunosorbent assay (ELISA). The ROC curve assay was used to analyze the preoperative serum MMP-9 level to determine the most valuable cut-off point. The relationship between MMP-9 and clinicopathological features of HC patients was analyzed. Kaplan-Meier method was used to analyze the prognostic factors, and COX regression model was used to analyze the independent risk factors affecting prognosis. Results: Preoperative serum MMP-9 levels were significantly elevated in the death patients compared with the survival patients. The most valuable cut-off point for preoperative serum MMP-9 for prognosis was 201.93 ng/mL. Preoperative serum MMP-9 was associated with Bismuth-Corlette classification) and lymph node metastasis. Kaplan-Meier analysis showed that MMP-9, Bismuth-Corlette classification, Lymph node metastasis, Portal vein invasion, Hepatic artery invasion, Liver invasion, Incised margin, and Preoperative biliary drainage were related to prognosis. Cox regression model confirmed that hepatic artery invasion, liver invasion, incised margin, and MMP-9 have the potential to independence predicate prognosis in HC patients. Conclusion: Preoperative serum MMP-9 has high predictive value for prognosis and is an independent influencing factor for the prognosis of patients with hilar cholangiocarcinoma.

Colangiocarcinoma. Evaluación por Tomografía Computada y Resonancia Magnética / Cholangiocarcinomas. Evaluation by Computed Tomography and Magnetic Resonance

Resumen El colangiocarcinoma (CC) es el segundo tumor primario maligno más frecuente del hígado. La mayor incidencia se registra en Asia y su pronóstico es poco alentador. La resección quirúrgica del tumor es el único tratamiento potencialmente curativo. Sin embargo, la mayoría de los CC se diagnostican en estadios avanzados de la enfermedad y la recurrencia del tumor es común. Este trabajo pretende demostrar las características imagenológicas de los CC, su clasificación, así como también la evaluación preoperatoria por tomografía computada (TC) y resonancia magnética (RM) que el radiólogo debe realizar actualmente.

Abstract The cholangiocarcinoma (CC) is the second most common malignant primary tumor of the liver. The highest incidence is registered in Asia and its prognosis is not very encouraging. The surgical resection is the only potentially curative treatment, however most of the CCs are diagnosed in advanced stages and the tumor recurrence is frequently presented. The purpose of this study is to show the imagenologic characteristics of CC, its classification as well as the preoperative evaluation by Computed Tomography (CT) and Magnetic Resonance (MR) that the radiologist must perform currently.

Derivação biliodigestiva no tratamento do tumor de Klatskin / Intestinal biliary bypass in Klatskin's tumor treatment

INTRODUÇÃO: Colangiocarcinomas são formas incomuns de neoplasia gastrointestinal, cuja incidência varia de 0,01% a 0,8%. Os tumores de Klatskin são colangiocarcinomas hilares originados na bifurcação do ducto hepático principal. Atualmente a maioria dos tratamentos é paliativa. OBJETIVOS: Relatar um caso de tumor de Klatskin submetido à drenagem cirúrgica da via biliar intra-hepática. RELATO DO CASO: Homem de 68 anos, procurou assistência médica com quadro ictérico intenso e colangiorressonância magnética confirmou o diagnóstico de tumor de Klatskin. Levado à laparotomia exploradora verificou-se massa em hilo hepático com invasão portal, hepática e do complexo duodenopancreático. Optou-se por derivação biliodigestiva e hepatectomia parcial esquerda com anastomose hepaticojejunal. Houve boa evolução no pós-operatório, com diminuição da icterícia. CONCLUSÃO: A derivação biliodigestiva no tratamento do tumor de Klatskin é procedimento que alivia o quadro ictérico melhorando a qualidade de vida, e pode ser utilizado quando há irressecabilidade tumoral.

INTRODUCTION: Cholangiocarcinomas are uncommon (0,01 to 0,8%) forms of gastrointestinal neoplasms. The Klatskin tumour is ductal cholangiocarcinoma originated on the bifurcation of main hepatic duct. Currently, the majority of cases are treated in palliative way. CASE REPORT: Man with 68 years old seek medical assistance with intense jaundice and MRI gave the final diagnosis of Klatskin tumour. Exploratory laparotomy showed big hepatic mass involving portal vein and neighboring tissues. He was submitted to palliative procedure with hepatojejunal anastomosis and partial hepatectomy. He was discharged from the hospital with better clinical conditions and alleviated from his jaundice. CONCLUSION: Intestinal biliary bypass in Klatskin is an indicated procedure to alleviate the jaundice in the terminal phase of this tumour, improving the quality of live.

Colangiocarcinoma: Actualización, diagnóstico y terapia: [revisón] / Cholangiocarcinoma: [review]

Cholangiocarcinoma is a malignant lesion of the bile duct epithelium. Its incidence and prevalence are low. It appears from the sixth decade of life and there is slight male predominance. It is most frequently found in the confluence of the hepatic ducts, where it is called hilar cholangiocarcinoma or Klatskin tumor. Its etiology is unknown but there are predisposing conditions and environmental risk factors such as primary sclerosing cholangitis, Caroli's disease, bile duct malformations, industrial toxins and parasitic infections. The classic presentation of cholangiocarcinoma includes jaundice, weight loss and right upper quadrant pain. These, in addition to laboratory exams, endoscopical and imaging procedures, lead to the diagnosis. Hilar cholangiocarcinoma must be distinguished from other malignant or benign causes of biliary obstruction. Cholangiocarcinoma of the distal common bile duct must be differentiated from other periampullary tumors and intrahepatic cholangiocarcinoma can be confused with a hepatocellular carcinoma. Two classifications are used for clinical staging: TNM and Bismuth-Corlette. The best treatment is the complete surgical excision with negative histological margins, although the resectability index is low. The type and size of surgery depends on the location and extent of the tumor. Patients with unresectable tumors can be subjected to palliative procedures such as biliary-enteric bypass, endoscopic or pecutaneous stent placement. Chemotherapy is not effective. Recently, endoscopic phototherapy has emerged as a better alternative for palliative care.

Traqueobroncopatia osteocondroplástica em portador de tumor de Klatskin: relato de caso e revisão da literatura / Tracheopathia osteochondroplastica in a patient with Klatskin tumor: a case report and review of the literature

A traqueopatia osteocondroplástica (TO) é uma desordem idiopática, incomum, caracterizada pela presença de nódulos osteocartilaginosos na submucosa das vias aéreas, causando rigidez e estreitamento da árvore respiratória. Afeta principalmente homens acima dos 50 anos com manifestações clínicas devidas à obstrução e/ou a infecções locais. Sua patogênese é desconhecida. Relatamos um caso de TO encontrada acidentalmente em autopsia de mulher com 73 anos de idade, que apresentava carcinoma ductal biliar extra hepático (tumor de Klatskin).

Tracheopathia osteochondroplastica (TO) is an unusual idiopathic disorder, characterized by osteocartilaginous nodules in the submucosa of the respiratory airway, which causes rigidity and narrowing of the respiratory tree. It affects mainly men over 50 and clinical manifestations are due to obstruction and/or local infections. The pathogenesis is uncertain. We report a case of TO found incidentally in an autopsy of a 73 year-old woman, who had an extrahepatic biliary ductal carcinoma (Klatskin tumor).

Mulher de 69 anos de idade com obstrução biliar / A 69-year-old woman with biliary obstruction

Relata-se o caso de uma mulher com 69 anos com obstrução biliar, para enfatizar desafios no diagnóstico e no tratamento do colangiocarcinoma hilar (tumor de Klatskin). Classificado como Bismuth-Corlette tipo II, o tumor poderia ser ressecado, mas o caso foi considerado inoperável, na ocasião do diagnóstico, devido a condições clínicas. Foram realizadas drenagens biliares paliativas (percutânea e endoscópica). O óbito ocorreu por insuficiência renal e choque séptico.

A 69-year-old woman presenting with biliary stricture is reported, to emphasize diagnosis and treatment challenges about hilar cholangiocarcinoma (Klatskin tumor). Classified as Bismuth-Corlette type II, the tumor could be resectable, but the patient was considered inoperable at occasion of diagnosis, due to her clinical conditions. Palliative biliary drainages (percutaneous transhepatic and endoscopic) were performed. She died due to renal failure and septic shock.